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1.
《Cor et vasa》2018,60(3):e331-e334
This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.  相似文献   

2.
Heart failure is a serious condition and equivalent to malignant disease in terms of symptom burden and mortality. At this moment only a comparatively small number of heart failure patients receive specialist palliative care. Heart failure patients may have generic palliative care needs, such as refractory multifaceted symptoms, communication and decision making issues and the requirement for family support. The Advanced Heart Failure Study Group of the Heart Failure Association of the European Society of Cardiology organized a workshop to address the issue of palliative care in heart failure to increase awareness of the need for palliative care. Additional objectives included improving the accessibility and quality of palliative care for heart failure patients and promoting the development of heart failure‐orientated palliative care services across Europe. This document represents a synthesis of the presentations and discussion during the workshop and describes recommendations in the area of delivery of quality care to patients and families, education, treatment coordination, research and policy.  相似文献   

3.
BACKGROUND: Advances in care have improved the longevity and quality of life for children with congenital heart disease; however, many of them need lifelong highly qualified specialist care. The cardiac lesion involved may not always be the patient's main problem; issues related to quality of life may dominate. AIM: To explore and gain a deeper understanding of young adults experiences of living with congenital heart disease in order to enhance the quality of care provided by the health care system. METHOD: Transcribed in-depth interviews were analysed using a phenomenological-hermeneutic method. Six adults with congenital heart disease, aged 22-39 years old, were included in the study. RESULTS: Analyses yielded two themes, having the disease and living with illness, both originating from the main theme of ambivalence. The interviewees were ambivalent in how they viewed themselves, how they faced their daily life and how they dealt with their encounters with the health care system. They had to strike a balance between being different and not being different; being sick and being healthy; revealing their congenital heart disease or hiding it and living with a hidden handicap. They also had to cope with the disease and with the health care system. CONCLUSION: Young adults with congenital heart disease are ambivalent. They have a strong wish to be healthy and they might hide their symptoms from the healthcare personnel and sometimes even from themselves. A psychosocial preparedness when meeting these patients is necessary.  相似文献   

4.
5.
Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children's hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease. Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school. Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available "off the rack", although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care. With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist. In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not "cured", but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.  相似文献   

6.
This report addresses the needs and problems of grown-up congenital heart (GUCH) patients and makes recommendations on organisation of national medical care, training of specialists, and education of the profession. The size of the national population of patients with grown-up congenital heart disease (GUCH) is uncertain, but since 80-85% of patients born with congenital heart disease now survive to adulthood (age 16 years), an annual increase of 2500 can be anticipated according to birth rate. Organisation of medical care is haphazard with only three of 18 cardiac surgical centres operating on over 30 cases per annum and only two established specialised units fully equipped and staffed. Not all grown-ups with congenital heart disease require the same level of expertise; 20-25% are complex, rare, etc, and require life long expert supervision and/or intervention; a further 35-40% require access to expert consultation. The rest, about 40%, have simple or cured diseases and need little or no specialist expertise. The size of the population needing expertise is small in comparison to coronary and hypertensive disease, aging, and increasing in complexity. It requires expert cardiac surgery and specialised medical cardiology, intensive care, electrophysiology, imaging and interventions, "at risk" pregnancy services, connection to transplant services familiar with their basic problem, clinical nurse specialist advisors, and trained nurses. An integrated national service is described with 4-6 specialist units established within adult cardiology, ideally in relation or proximity to university hospital/departments in appropriate geographic location, based in association with established paediatric cardiac surgical centres with designated inpatient and outpatient facilities for grown-up patients with congenital heart disease. Specialist units should accept responsibility for educating the profession, training the specialists, cooperative research, receiving patients "out of region", sharing particular skills between each other, and they must liaise with other services and trusts in the health service, particularly specified outpatient clinics in district and regional centres. Not every regional cardiac centre requires a full GUCH specialised service since there are too few patients. Complex patients need to be concentrated for expertise, experience, and optimal management. Transition of care from paediatric to adult supervision should be routine, around age 16 years, flexibly managed, smooth, and explained to patient and family. Each patient should be entered into a local database and a national registry needs to be established. The Department of Health should accept responsibility of dissemination of information on special needs of such patients. The GUCH Patients' Association is active in helping with lifestyle and social problems. Easy access to specialised care for those with complex heart disease is crucial if the nation accepts, as it should, continued medical responsibility to provide optimal medical care for GUCH patients.  相似文献   

7.
AIMS: To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. METHODS AND RESULTS: Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions ('defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common. CONCLUSION: The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period.  相似文献   

8.

BACKGROUND:

Pediatric institutions play a large role in preparing young adults with congenital heart disease to transition to adult care.

OBJECTIVE:

To determine the perspectives of patients, parents and providers on transition preparation.

METHODS:

Patients aged nine to 18 years with congenital heart defects and their respective parent(s) participated in semistructured interviews. Health care providers completed a self-administered survey.

RESULTS:

A total of 23 patients, 22 parents and 45 health care providers were enrolled in the study. Only 36% of patients demonstrated a clear understanding of transition and its implications for their cardiac care. Parents were extensively involved in care activities, with 95% accompanying their child to visits at the clinic, 68% staying with their child for the entire visit and 45% administering their medication. Children more knowledgeable about their diagnosis demonstrated a better understanding about their transition to adult care (100% versus 7%, respectively; P<0.01) and were more likely to communicate directly with their providers than those who were less or not knowledgeable (88% versus 33%, respectively; P=0.03). Nurses were more likely than physicians to view increased parental involvement in care activities as a barrier to transition preparation (37% versus 5%, respectively; P=0.02).

CONCLUSIONS:

A lack of clear role expectations indicates a need for the pediatric health care setting to adapt appropriately to address the shifting needs of adolescent and young adult patients and their families. A formalized approach to transition preparation for adolescents with congenital heart disease needs to emphasize comprehensive education. A delegation of explicit responsibilities and the clear definition of roles for parents, providers and patients are necessary to provide young adults with the resources and support necessary to achieve a successful transition to adult care.  相似文献   

9.
Over the next 10 years, heart failure is likely to become a medical and sociological problem as a result of improved treatment of ischaemic heart disease and hypertension. At present, in Poland, there are only 50% of the cardiological or cardiac surgery procedures (coronarography, PTCA, CABG, surgery of congenital or acquired heart disease) performed compared to Western Europe. After being registered on the waiting list, it can take anything between 3 and 12 months before the procedure is done. Patients with heart failure have diagnostic tests such as ECG, chest X-ray, and biochemical evaluation performed regardless of the level of care. When echocardiography, exercise testing or Holter monitoring is required, it is done at specialist or reference specialist facilities with a waiting time of approximately 1-3 months. Pharmaceutical treatment of CHF is also inadequate. ACE inhibitors are prescribed in approximately 68% of patients. The average prescribed dosage is far from that recommended in guidelines. Only 18-29% of patients with HF are on beta blockers. The improvement of cardiological care standards depends mainly on the financial resources of State Health System Agencies.  相似文献   

10.
Heart failure (HF) constitutes the growing cardiovascular burden and the major public health issue, but comprehensive statistics on HF epidemiology and related management in Europe are missing. The Heart Failure Association (HFA) Atlas has been initiated in 2016 in order to close this gap, representing the continuity directly rooted in the European Society of Cardiology (ESC) Atlas of Cardiology. The major aim of the HFA Atlas is to establish a contemporary dataset on HF epidemiology, resources and reimbursement policies for HF management, organization of the National Heart Failure Societies (NHFS) and their major activities, including education and HF awareness. These data are gathered in collaboration with the network of NHFS of the ESC member and ESC affiliated countries. The dataset will be continuously improved and advanced based on the experience and enhanced understanding of data collection in the forthcoming years. This will enable revealing trends, disparities and gaps in knowledge on epidemiology and management of HF. Such data are highly needed by the clinicians of different specialties (aside from cardiologists and cardiac surgeons), researchers, healthcare policy makers, as well as HF patients and their caregivers. It will also allow to map the snapshot of realities in HF care, as well as to provide insights for evidence‐based health care policy in contemporary management of HF. Such data will support the ESC/HFA efforts to improve HF management ant outcomes through stronger recommendations and calls for action. This will likely influence the allocation of funds for the prevention, treatment, education and research in HF.  相似文献   

11.
《Congenital heart disease》2017,12(3):242-250
The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease‐specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients.  相似文献   

12.
Use of angiotensin-converting enzyme inhibitor in heart failure with left ventricular systolic dysfunction has revolutionized management of heart failure and resulted in several national heart failure guidelines. However, studies have documented underutilization of these life saving drugs. Subsequently, results of other randomized clinical trials revealed the beneficial effects of angiotensin receptor blockers, beta-blockers, and spironolactone in heart failure with systolic dysfunction, which prompted several other guidelines. However, all these guidelines were primarily restricted to systolic dysfunction. Heart failure is as much a geriatric syndrome as it is a cardiac. Most heart failure patients are older adults. About half of the heart failure in older adults is not associated with left ventricular systolic dysfunction. Two recent heart failure guidelines, from the European Society of Cardiology and the American College of Cardiology/American Heart Association, published respectively in September and December 2001 included recommendations for patients with preserved left ventricular systolic function and other special populations, including older adults and those at end of life. The purpose of this article is to review the relevance of these two guidelines to heart failure patients living in long-term care facilities. As our population is aging, more heart failure patients are expected to live in long-term care facilities. Appropriate evaluation and management of these patients are essential to improve quality of heart failure care in the coming decades.  相似文献   

13.
Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
Design: A retrospective cohort study.
Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
Interventions: We analyzed for protective factors associated with age‐appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities.
Outcome Measures: We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008‐2015.
Results: Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months be‐ tween last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe).
Conclusion: Patients with severe disease are more likely to transfer to adult congeni‐ tal heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to under‐ stand and address barriers in order to improve successful transfer.  相似文献   

14.
There is a increasing clinical and scientific interest in adults with congenital heart disease. Germany is among, if not the first country worldwide, with a structured care of adults with congenital heart disease. Due to the advances in cardiology and in heart surgery, the primarily high mortality rate of relevant congenital heart disease has declined from 80% to less than 20% during the recent decades. Currently, in Europe the estimated number of adults with congenital heart disease is between 1,2 and 2,7 Mio., and the number in Germany exceeds 180.000. Almost all congenital heart defects, either native or interventionally or surgically treated, need regular control, since even after successful primary treatment significant residua or sequels have to be expected. Optimal care of these patients begins in the childhood and must be continued down to the high adulthood. The complexity of congenital heart defects makes a multidisciplinary approach necessary. A specialized, interdisciplinary cooperation between cardiologists, pediatric cardiologists, cardiac surgeons and other specialists is mandatory.  相似文献   

15.
In 2008 the 10 Principles of Haemophilia Care were outlined to provide a benchmark for haemophilia treatment. The EHTSB performed a survey to establish to what extent the Principles of Haemophilia Care were being applied throughout Europe. In total, 21 centres from 14 countries (France, UK, Germany, Switzerland, Sweden, Norway, the Netherlands, Belgium, Poland, Portugal, Slovakia, Spain, Greece and Italy), were surveyed. A central organization of haemophilia care (principle 1) was present in 79%, and a central patient registry in 57% (principle 2). National haemophilia care decision‐making was performed by clinicians, ministries and patient organizations (principle 4). All had designated comprehensive care centres (CCC – principle 3), responsible the majority of severe patients, but in 36% some patients were treated outside CCC/haemophilia treatment centres (HTC)s. Clotting factor concentrates were available everywhere, without dosing restraints (principle 5), including recombinant products in 86% of countries. Prophylactic treatment was available for all children but not for all adults (principle 7). Immune tolerance was available in all countries (principle 9). Home treatment was supported and taught by all centres (principle 6). At centre level, 86% had 24‐h laboratory facilities and all participated in education and research (principle 10). An experienced haematologist was available at all centres, a paediatrician in 47%, and prompt out of hours review was available in all (principle 8). The Principles of Haemophilia Care were generally applied throughout Europe. Some aspects of centralization, national organization of care, use of registries, formal paediatric care and prophylaxis for adults may be improved.  相似文献   

16.
Establishing paediatric cardiology service in a country with limited resources like Sudan is a challenging task.A paediatric cardiac team was formed then the services in different disciplines were gradually established. Echocardiography (echo) clinics were founded in tertiary and peripheral hospitals. Cardiac catheterization (cath) was established at the Sudan Heart Centre (SHC) in 2004 and over 400 procedures had been performed including interventional catheterization like pulmonary valve dilatation, patent ductus arteriosus and atrial septal defect device closure.Congenital heart surgery started in 2001, currently 200 cases are done each year including closed procedures as well as open heart procedures for patients weighing more than 8 kg. Cardiology-cardiac surgery as well as adult congenital heart disease meetings were held and contributed positively to the services. The cardiology-cardiac surgery scientific club meeting was founded as a forum for academic discussions. A fellowship program was established in 2004 and included seven candidates trained in paediatric cardiology and intensive care. Two training courses had been established: congenital heart disease echo and paediatric electrocardiogram interpretation. Links with regional and international cardiac centres had important roles in consolidating our program.Significant obstacles face our service due to the small number of trained personnel, high cost of procedures, the lack of regular supplies and lack of cardiac intensive care facilities for young infants.Bridging the huge gap needs extensive official as well as non-governmental efforts, training more staff, supporting families and collaboration with regional and international centres.  相似文献   

17.
18.
Objective: Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better under‐ stand the patterns and influences on end‐of‐life care intensity for adults with con‐ genital heart disease.
Methods: We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi‐hospital health care system. We used multi‐ variate analysis to evaluate markers of resource utilization, location of death, and documentation of advance care planning among patients with congenital heart dis‐ ease versus those with cancer and those with heart failure.
Results: Approximately 40% of adults with congenital heart disease experienced in‐ patient and intensive care unit (ICU) hospitalizations in the last 30 days of life; 64% died in the hospital. Compared to patients with cancer, patients with adult congenital heart disease (ACHD) were more likely to have inpatient (adjusted risk ratio 1.57; 95% CI 1.12‐2.18) and ICU admissions in the last 30 days of life (adjusted risk ratio 2.56; 95% CI 1.83‐3.61), more likely to die in the hospital (adjusted risk ratio 1.75; 95% CI 1.43‐2.13), and more likely to have documentation of advance care planning (adjusted risk ratio 1.46; 95% CI 1.09‐1.96). Compared to patients with heart failure (HF), patients with ACHD were less likely to have an ICU admission in the last 30 days of life (adjusted risk ratio 0.73; 95% CI 0.54‐0.99).
Conclusions: Adults with congenital heart disease have significant hospital resource utilization near the end of life compared to patients with cancer, notable for more hospitalizations and a higher likelihood of death in the hospital. This population rep‐ resents an important opportunity for the application of palliative and supportive care.  相似文献   

19.
The demography of congenital heart disease is changing. Largely as a consequence of successful cardiac surgery in childhood, there are an increasing number of adults with congenital heart disease with a prevalence of more than four per 100 adults. The type of disease in adults is also changing with an increasing number of survivors with complex disease. These patients have a significantly increased healthcare requirement in comparison to healthy adults and this includes noncardiac, multisystem morbidity. The adult congenital heart disease population are now developing problems associated with aging and there is a new population of geriatrics with congenital heart disease. As survival continues to improve, increased healthcare resources need to be directed towards the management of the adult with congenital heart disease.  相似文献   

20.
It is well established that organized care of heart failure patients, including specialist management by cardiologists, improves patient outcomes. In response to this, other national training bodies (the UK and the USA) have developed heart failure subspecialty curricula within their Cardiology Training Curricula. In addition, European Society of Cardiology (ESC) subspecialty curricula exist for Interventional Cardiology and Heart Rhythm Management. The purpose of this heart failure curriculum is to provide a framework which can be used as a blueprint for training across Europe. This blueprint mirrors other ESC curricula. Each section has three components: the knowledge required, the skills which are necessary, and the professionalism (attitudes and behaviours) which should be attained. The programme is designed to last 2 years. The first year is devoted to the specialist heart failure module. The second year allows completion of the optional modules of advanced imaging, device therapy for implanters, cardiac transplantation, and mechanical circulatory support. The second year can also be devoted to continuation of specialist heart failure training and/or research for those not wishing to continue with the advanced modules.  相似文献   

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